BackgroundDiagnostic and surgical management of severe chronic angle- closure glaucoma secondary to ciliary body cysts can be difficult to manage in a patient with oculodentodigital dysplasia.Case presentationA 6-year old girl with oculodentodigital dysplasia, with progressive chronic angle- closure glaucoma secondary to ciliary body cysts presented to our clinic. The initial examination revealed counting fingers vision in the left eye. Intraocular pressure (IOP), as assessed by tonopen, was 31 mm Hg. Ultrasound biomicroscopy revealed ciliary body cysts in the left eye, and gonioscopy confirmed chronic angle closure. A tube shunt was placed to control the elevated IOP. A year after her tube shunt placement in the left eye, ultrasound biomiscropy was performed on her right eye and showed no ciliary body cysts. Gonioscopy in the right eye revealed an open angle to the ciliary body band. Subsequent serial gonioscopy every 3 months showed gradual narrowing of the right eye angle and finally three-and-a-half years after tube placement of the left eye, her right eye IOP became uncontrolled with medications alone and a tube shunt was similarly placed in the right eye. Intraoperative ultrasound biomicroscopy performed at the time of the right eye tube shunt revealed extensive ciliary body cysts in the right eye. Her IOP in both eyes have been well controlled since the placement of tube shunts.ConclusionsThis is one of the first reported cases of severe chronic angle- closure glaucoma secondary to ciliary body cysts in a patient with oculodentodigital dysplasia. We believe that early screening for ciliary body cysts is important in patients with oculodentodigital dysplasia.

BackgroundSixth (abducens) nerve palsy (ANP) is far less frequent in children and has not been reported as a sign of acute disseminated encephalomyelitis (ADEM). We present an infant case of ADEM with bilateral abducens nerve palsy (BANP).Case presentationWe report one case of BANP in a 15-month-old boy of fulminant ADEM. The patient underwent physical examinations and brain MRI scan three times during about six months follow-up. The patient had BANP and developmental regression when he regained consciousness from a coma, and the signs had persisted for 6-months.ConclusionsBANP can be a symptom of ADEM.

BackgroundThis study evaluated the anterior ocular segment in a pseudophakic eye with angle closure due to a plateau-like iris associated with Soemmering’s ring, using ultrasound biomicroscopy (UBM) and anterior segment optical coherence tomography (AS-OCT).Case presentationA 60-year-old woman was referred from a local clinic due to sudden-onset ocular pain and uncontrolled intraocular pressure (IOP) in the left eye, which was 56 mmHg after treatment with latanoprost, timolol, and dorzolamide eye drops. Fourteen years earlier, she developed acute primary angle closure. At that time, because the IOP remained elevated after a peripheral iridectomy, cataract extraction combined with goniosynechialysis was added. After the IOP decreased to within the normal range, a secondary intraocular lens was implanted outside the bag. On this admission, UBM and AS-OCT images showed angle closure caused by the combination of a plateau-like iris and contact between the mydriatic pupillary margin and enlarged Soemmering’s ring. After adding 2 % pilocarpine four times a day, the mydriasis resolved slightly, and the IOP decreased to the normal range between 8 and 18 mmHg. AS-OCT images showed re-opening of the angle structure after treatment with 2 % pilocarpine.ConclusionThe intraocular pressure and angle structure in eyes with a plateau iris after cataract extraction should be followed carefully.

BackgroundAngiomyolipoma is a benign mesenchymal tumor composed of variable amounts of smooth muscle, adipose tissue and thick-walled blood vessels, and usually named PEComas (perivascular epithelioid cell tumors). PEComas share overlapping histopathological features with epithelioid cells along a perivascular distribution and characteristic immunohistochemistry with coexpression of myoid and melanocytic markers (HMB-45 /or Melan-A). We report the first case of primary orbital angiomyolipoma with negative melanocytic marker.Case presentationAn 80-year-old Asian woman had a 2-year history of progressive swelling in the left upper eyelid. External examination revealed 3 cm of relative proptosis of the left eye and a palpable mass in the left superonasal orbit. Computed tomographic scan demonstrated a circumscribed, heterogeneous orbital mass. Excision biopsy was done and the histological finding demonstrated the orbital mass was composed of mature adipocytes, intermingled with spindle or oval-shaped cells, and accompanied by thick-walled blood vessels. Immunohistochemically, tumor cells were positive for CD34 and HHF-35, but negative for cytokeratin, HMB-45 and Melan-A. The diagnosis of angiomyolipoma was made. No recurrence was noted at 2-year follow-up.ConclusionIn our case, the HMB-45 negativity may be explained by the rarity of the epithelioid cells, and the HMB-45 positivity is often weaker or absent in spindle cells. Angiomyolipoma, although rare, should be added to the differential diagnosis of space-occupying orbital lesion.

BackgroundSubcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare, highly malignant, extranodal lymphoma that preferentially infiltrates into subcutaneous adipose tissue. No case of SPTCL with the earliest symptoms occurring in the eye region has been reported. We report a case of SPTCL complicated by eyelid swelling.Case presentationA 19-year-old Japanese man presented with worsening left eyelid swelling. The patient’s best-corrected visual acuity (BCVA) was 0.8, left intraocular pressure was 36 mm Hg, and he had prominent mucosal hyperemia and edema. His left eye had light reflex attenuation and a positive relative afferent pupillary defect, but no abnormality in the visual field or central flicker value. Magnetic resonance imaging showed left orbital adipose tissue inflammation. The blood examination was normal. He was hospitalized for an intensive examination and treatment for possible cellulitis, orbital panniculitis, and inflammatory pseudotumor. Systemic antibiotics were initiated. The following day, he underwent a sub-Tenon’s injection of triamcinolone. Left eyelid swelling gradually improved. He was discharged on the ninth day and followed up with oral prednisolone. Two months later, he visited our department because of a high fever and slight right eyelid swelling. Ocular hypertension was detected. A blood examination revealed pancytopenia. Computed tomography showed fluid retention, hydrothorax, and abdominal dropsy. Magnetic resonance imaging revealed right orbital panniculitis. Because of suspected hemodyscrasia, he was referred to the hematology department of another hospital where he was diagnosed with SPTCL.ConclusionsThe possibility of SPTCL, with attention to recurrence and systemic symptoms, should be considered in young patients with sudden eyelid swelling.

BackgroundThe ocular presentation of Castleman’s disease (CD)-associated paraneoplastic pemphigus (PNP) has rarely been reported. In this report, we describe a young patient with CD-associated PNP who had recurrent corneal ulceration in addition to cicatrizing conjunctivitis.Case presentationWe describe a case of 23-year-old male with mucocutaneous erosion and conjunctival injection and erosion who was found to have PNP. Pelvic hyaline-vascular CD was detected and completely excised. The mucocutaneous lesions improved postoperatively. Two years after pelvic surgery, the patient gradually developed conjunctival symblepharon in both eyes and pterygium in the right eye. The patient then underwent a successful exclusion of the symblepharon, an excision of the pterygium and an amniotic membrane transplantation in the right eye. However, after 6 months, he experienced an aseptic corneal ulcer and recurrent pterygiumin the right eye. After treatment with systemic and local immunosuppressive medications, the corneal ulcer gradually healed and remained stable.ConclusionCorneal ulceration and melting, in addition to conjunctivitis, as a complication of CD-associated PNP, can be successfully managed with systemic and local immunosuppressants.